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JuhaSinisalo

Takotsubo Cardiomyopathy

Essentials

  • Initially, takotsubo cardiomyopathy resembles a myocardial infarction: there is chest pain and dyspnoea.
  • The symptoms usually begin after an episode of severe emotional or physical stress. In about 1 out of 4 patients, no clear triggering factor can be identified.
  • The patients are typically HASH(0x2ed2410) 60-year-old women.
  • They should be hospitalized.
  • Initial treatment should be given according to the guidelines for myocardial infarction because the two are difficult to distinguish.
  • Once the diagnosis of takotsubo cardiomyopathy has been confirmed, treatment is similar to that of cardiac failure.
  • Most patients recover completely within about a month but slightly less than 10% have complications, i.e. die or have a cerebral infarction.

Prevalence

  • Of all patients with symptoms of myocardial infarction, about 2% have takotsubo cardiomyopathy;
  • 90% of these are women.
  • In Finland (population 5.5 million), about 350 new cases of takotsubo cardiomyopathy are diagnosed per year.

Aetiology and pathophysiology

  • The exact aetiology of takotsubo cardiomyopathy is unknown.
  • Acute stress or excessive physical exertion is believed to activate the central and autonomic nervous systems. This elevates cortisol and catecholamine levels in the body.
  • The myocardium is damaged by the direct toxic effect of the catecholamines and, on the other hand, by signals mediated by adrenergic receptors.
  • Microvascular spasm is believed to be involved.
  • The patient typically develops acute akinesis in the apex of the left ventricle, producing a shape resembling a squid trap (tako tsubo in Japanese). This results in acute cardiac failure.
  • The akinesis leads to insufficient pumping, predisposing to formation of a blood clot in the apex of the heart, which may later lead to cerebral infarction.

Symptoms

  • The symptoms resemble myocardial infarction.
  • Three in four patients have chest pain and one in two suffer from dyspnoea.
  • Syncope occurs in a small share of patients.
  • Patients may be asymptomatic at first.

Diagnosis

Diagnostic criteria

  • As takotsubo cardiomyopathy cannot be reliably distinguished from myocardial infarction, it must be treated like myocardial infarction.
  • The patients are usually postmenopausal women lacking the typical risk factors of coronary artery disease.
  • ECG changes (ST elevation, ST depression, T inversion) resemble those seen in myocardial infarction or, sometimes, myocarditis.
    • In 2 cases out of 3, the ECG resembles that seen in ST elevation MI. T inversions can be seen in 2 of 3 cases and Q waves in 1 out of 3 cases.
    • Extensive ST changes may resemble those seen in myocarditis.
    • In some cases, ECG changes may be minor.
  • Troponin concentration is similarly increased as in myocardial infarction.
  • Echocardiography may reveal typical akinesis in the apical area but this may be difficult to distinguish from myocardial infarction.

Confirming the diagnosis

  • Contrast study of the coronary arteries and the left ventricle is needed to confirm the diagnosis.
    • This usually confirms the diagnosis
    • Constrictive coronary artery disease does not rule out takotsubo cardiomyopathy, since it can be found in about 1 out of 4 patients with takotsubo cardiomyopathy.
  • In some cases, the diagnosis can only be confirmed after cardiac MRI.

Treatment

Initial treatment

  • Patients should be hospitalized.
  • The symptoms resemble those of myocardial infarction - initial treatment should be chosen based on symptoms and ECG findings.
  • If the symptoms resemble non-ST-elevation myocardial infarction, treatment should be chosen accordingly (ASA, LMWH and haemodynamic stabilization).
  • If ECG shows an ST elevation MI, proceed according to the relevant guidelines (ASA, LMWH, and, according to local guidelines, ADP receptor blocker, haemodynamic stabilization and immediate referral to a unit performing emergency angiography).

Drug treatment after the initial phase

  • Once the diagnosis of takotsubo cardiomyopathy has been confirmed, treatment is similar to that of cardiac failure.
  • The ADP receptor blocker and ASA can be withdrawn.
  • An ACE inhibitor and a beta-blocker should be started and these continued until ventricular function has normalized.
  • LMWH should be continued at least until the end of hospital treatment, preferably for at least 10 days.
  • If there are signs of a blood clot at the apex of the heart or if the ventricular apex is severely akinetic, longer-term anticoagulant therapy (warfarin/DOAC) should be started.
    • MRI may be useful for making the decision.

Follow-up

  • An appointment should be made for a check-up 4 to 6 weeks after the attack to confirm by echocardiography that cardiac failure can no longer be seen.
    • If this is so, the beta-blocker, ACE inhibitor and any anticoagulant therapy can be withdrawn.

    References

    • Templin C, Ghadri JR, Diekmann J et al. Clinical Features and Outcomes of Takotsubo (Stress) Cardiomyopathy. N Engl J Med 2015;373(10):929-38. [PubMed]
    • Scally C, Rudd A, Mezincescu A et al. Persistent Long-Term Structural, Functional, and Metabolic Changes After Stress-Induced (Takotsubo) Cardiomyopathy. Circulation 2018;137(10):1039-1048. [PubMed]
    • Lyon AR, Citro R, Schneider B ym. Pathophysiology of Takotsubo Syndrome: JACC State-of-the-Art Review. J Am Coll Cardiol 2021;77(7):902-921. [PubMed]